Issue: August 2023
Fact checked byRichard Smith

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June 19, 2023
3 min read
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Adults diagnosed with adrenocortical cancer have 54% overall survival at 5 years

Issue: August 2023
Fact checked byRichard Smith
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Key takeaways:

  • Of a cohort of adults with adrenocortical cancer, 38.1% died during a median follow-up of 30 months.
  • Incidental cancer was associated with a longer overall survival than symptomatic disease.

Adults who are diagnosed with adrenocortical cancer have a 5-year overall survival of 54%, with overall survival higher among those with stage I or stage II cancer, according to study findings.

In a retrospective analysis of data from 512 adults with adrenocortical cancer, researchers analyzed presentation characteristics, treatment strategies and associations with overall survival. Of the cohort, 38.1% died during a median follow-up of 30 months, and adults with localized disease who were older, had stage III cancer, cortisol secretion, cancer recurrence or higher Ki-67 response had a higher risk for death.

Risk factors for adrenocortical cancer among adults.
Data were derived from Puglisi S, et al. J Clin Endocrinol Metab. 2023;doi:10.1210/clinem/dgad199.

“Adrenocortical cancer is a sex-related disease and demonstrates that both tumor characteristics and treatment strategies have an impact on clinical outcome,” Soraya Puglisi, MD, PhD, of the department of clinical and biological sciences, division of internal medicine at San Luigi Hospital, University of Turin in Italy, and colleagues wrote in a study published in The Journal of Clinical Endocrinology & Metabolism. “We demonstrate for the first time that incidental adrenocortical cancer may have a better prognosis than symptomatic tumors and that disease recurrence is a strong predictor of mortality.”

Researchers obtained data from 512 adults with adrenocortical cancer (60.2% women; median age, 48 years) who were evaluated from January 1990 to June 2018 at 12 tertiary centers in Italy. Demographics, cancer characteristics, treatment approach, recurrence date, date of last follow-up and mortality data were collected.

Of the cohort, adrenocortical cancer was an incidental finding for 38.1%. The percentage of adults with incidental cancer ranged from less than 20% among those younger than 30 years to more than 50% among those older than 70 years.

Among adults with localized disease, surgery was the first-line treatment in all cases, with open surgery performed in 72% of cases. Open surgery was more common for stage II and stage III cancers, whereas laparoscopic surgery was more common for stage I cancer. After radical surgical resection, 62.7% of adults received adjuvant therapy with mitotane (Lysodren, Bristol Myers Squibb). Mitotane therapy was prescribed for 45% of those with stage I cancer, 62% with stage II cancer and 73% with stage III cancer.

Among adults with stage IV metastatic disease, 70.3% received surgery. Open surgery was performed in 63.3% of those receiving surgery. Medical treatment was initiated for 88.1% of adults with metastatic disease. Of those receiving medical treatment, 76.4% received a combination of mitotane and chemotherapy, 14.6% received mitotane alone and 9% received chemotherapy alone.

“These findings highlight the value of surgery and adjuvant mitotane to prevent recurrence and improve patient outcome,” the researchers wrote. “Open surgery appears to be the most effective way of removing adrenocortical cancer.”

Recurrence was observed in 56.2% of adults during a median follow-up of 15 months. Recurrence-free survival at 5 years was 33% overall, 59% with stage I cancer, 36% with stage II cancer and 16% with stage III cancer.

Of the cohort, 38.1% died during a median follow-up of 30 months. Overall survival at 5 years was 54% for the full cohort, 71% with stage I cancer, 68% with stage II cancer, 41% with stage III cancer and 13% for stage IV cancer. Overall survival was longer for adults with incidental cancer compared with those who had local symptoms or hormone excess.

For those with localized disease, the risk for death was higher among older adults (HR = 1.04; 95% CI, 1.01-1.07; P = .003), those with stage III cancer (HR = 2.06; 95% CI, 1.27-3.36; P = .004), cortisol secretion (HR = 2.15; 95% CI, 1.34-3.46; P = .002) and higher Ki-67 response (HR = 1.02; 95% CI, 1.01-1.04; P = .004). Among adults with metastatic disease, cortisol secretion (HR = 2.05; 95% CI, 1.06-3.98; P < .05) and number of metastatic organs (HR = 1.77; 95% CI, 1.26-2.5; P < .001) were associated with a higher risk for death.

“The present study provides information on the management of patients with adrenocortical cancer in Italy, which could be used as a benchmark for future studies,” the researchers concluded.